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Pheochromocytoma-Induced Inverted Takotsubo-Like Cardiomyopathy Leading to Cardiogenic Shock Successfully Treated With Extracorporeal Membrane Oxygenation Benjamin Flam, Michael Broomé, Björn Frenckner, Robert Bränström and Max Bell J Intensive Care Med published online 6 October 2014 DOI: 10.1177/0885066614552992 The online version of this article can be found at: http://jic.sagepub.com/content/early/2014/10/01/0885066614552992

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Article

Pheochromocytoma-Induced Inverted Takotsubo-Like Cardiomyopathy Leading to Cardiogenic Shock Successfully Treated With Extracorporeal Membrane Oxygenation

Journal of Intensive Care Medicine 1-8 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0885066614552992 jic.sagepub.com

Benjamin Flam, MD1, Michael Broome´, MD, PhD2,3, Bjo¨rn Frenckner, MD, PhD2,4,5, Robert Bra¨nstro¨m, MD, PhD6,7, and Max Bell, MD, PhD1,3

Abstract Pheochromocytoma classically displays a variety of rather benign symptoms, such as headache, palpitations, and sweating, although severe cardiac manifestations have been described. We report a case of pheochromocytoma-induced inverted takotsubo-like cardiomyopathy leading to shock and cardiac arrest successfully treated with extracorporeal membrane oxygenation (ECMO) as a bridge to pharmacological therapy and curative adrenalectomy. A previously healthy 46-year-old woman presented to the emergency department with abdominal pain, dyspnea, nausea, and vomiting. Clinical evaluation revealed cardiorespiratory failure with hypoxia and severe metabolic acidosis. Computed tomography (CT) scan showed pulmonary edema and a left adrenal mass. Transthoracic echocardiography (TTE) displayed severe left ventricular dysfunction with inverted takotsubo contractile pattern. Despite mechanical ventilation and inotropic and vasopressor support, asystolic cardiac arrest ensued. The patient was resuscitated using manual chest compressions followed by venoarterial ECMO. Repeated TTEs demonstrated resolution of the cardiomyopathy within a few days. Laboratory results indicated transient renal and hepatic dysfunction, and CT scan of the brain displayed occipital infarctions. Biochemical testing and radionuclide scintigraphy confirmed a pheochromocytoma. Pharmacological adrenergic blockade was instituted prior to delayed adrenalectomy after which the diagnosis was histopathologically verified. The patient recovered after rehabilitation. We conclude that pheochromocytoma should be considered in patients presenting with unexplained cardiovascular compromise, especially if they display (inverted) takotsubo contractile pattern. Timely, adequate management might involve ECMO as a bridge to pharmacological therapy and curative surgery. Keywords pheochromocytoma; catecholamines; takotsubo cardiomyopathy; shock, cardiogenic; heart arrest; extracorporeal membrane oxygenation

1

Department of Anesthesiology and Intensive Care, Karolinska University Hospital, Solna, Stockholm, Sweden ECMO Department, Karolinska University Hospital, Stockholm, Sweden 3 Section of Anesthesiology and Intensive Care, Department of Physiology and Pharmacology, Karolinska Institutet, Stockholm, Sweden 4 Department of Pediatric Surgery, Astrid Lindgren Children’s Hospital, Karolinska University Hospital, Stockholm, Sweden 5 Division of Pediatric Surgery, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden 6 Department of Breast and Endocrine Surgery, Karolinska University Hospital, Stockholm, Sweden 7 Endocrine and Sarcoma Surgery Unit, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden 2

Received April 11, 2014, and in revised form May 29, 2014. Accepted for publication June 13, 2014. Corresponding Author: Benjamin Flam, Department of Anesthesiology and Intensive Care, Karolinska University Hospital, Solna, SE-171 76 Stockholm, Sweden. Email: [email protected]

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Journal of Intensive Care Medicine

Figure 1. Initial electrocardiogram was interpreted as sinus tachycardia with left anterior fascicular block without any apparent signs of ischemia, although baseline wandering made analysis of ST-segments difficult.

Introduction Pheochromocytoma is a rare, catecholamine-producing tumor of the chromaffin cells of the adrenal medulla (80%-85%) or extraadrenal tissues (ie, paraganglioma; 15%-20%).1 The annual incidence is estimated to be 2 to 8 cases per million population.2 It classically displays a variety of clinical manifestations including paroxysmal or sustained hypertension, palpitations, diaphoresis, and episodic headaches. However, it may occasionally manifest as noncardiogenic pulmonary edema,3 acute renal failure,4 acute myocardial infarction,5 myocarditis,6 or other forms of acute cardiomyopathy,7-9 with or without cardiogenic shock.10,11 Takotsubo cardiomyopathy is characterized by transient left mid-ventricular and often apical dysfunction and ballooning with sparing of basal segments in the absence of acute coronary artery obstruction.12 Inverted or reverse forms, involving basal segments with preserved contractility of the apex, have more recently been described.13 Although incompatible with a proposed diagnosis definition (Mayo Clinic modified criteria),14 its co-occurrence with pheochromocytoma has repeatedly been reported.8,15-19 Herein, we report the use of extracorporeal membrane oxygenation (ECMO) as a bridge to medical treatment followed by delayed curative adrenalectomy in a previously healthy woman presenting with pheochromocytoma-induced inverted takotsubolike cardiomyopathy leading to cardiogenic shock.

Case Presentation A 46-year-old woman of Turkish–Assyrian origin with no previous medical history presented to the emergency department

with acute onset of abdominal pain moving to the back, shortness of breath, nausea, and vomiting. She reported having an antecedent self-terminating episode of abdominal pain 4 days prior to admission. The family history contained unexplained sudden death of 2 aunts but no cases of neuroendocrine tumors. The patient denied use of tobacco or alcohol, nor did she take any medications or recreational drugs and did not have any known allergies. On admission, she was mentally obtunded and pale. She displayed acrocyanosis and had a respiratory rate of 40 breaths/min; oxygen saturation by pulse oximetry was immeasurable. Clinical examination revealed bilateral inspiratory crackles; her pulse was regular with a heart rate of 140 beats/min, and blood pressure was 125/90 mm Hg in the left arm and 120/90 mm Hg in the right arm. Her abdomen was soft on palpation with diffuse epigastric tenderness without palpable organomegaly or mass. She had no focal neurological deficit. Her body temperature was 35.7 C. Initial electrocardiogram was interpreted as sinus tachycardia with no signs of ischemia (Figure 1). Arterial blood gas analysis revealed severe hypoxemia (PaO2, 34 Torr [4.6 kPa]; SaO2, 39% [supplementary oxygen delivery of 15 L/min through a nonrebreather mask]) and metabolic acidosis (pH, 6.99; PaCO2, 42 Torr [5.6 kPa]; bicarbonate, 8 mmol/L; lactate, 18 mmol/L; base deficit, 24 mmol/L). Urinalysis was unremarkable. Further laboratory studies showed hyperglycemia (glucose, 24 mmol/L) and mild renal insufficiency (creatinine, 160 mmol/L). The patient was administered Ringer’s acetate and intravenous morphine. Blood and urinary cultures were obtained, and she was started on empiric broad-spectrum antibiotic therapy for suspected sepsis. A thoracoabdominal computed tomography (CT) scan showed bilateral lung consolidation suggestive of pulmonary

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Figure 2. Admission thoracoabdominal computed tomography scan with intravenous contrast showed bilateral lung consolidation suggestive of pulmonary edema or acute respiratory distress syndrome (A) and a highly vascularized 6.5  6.5  7.5 cm left adrenal mass (B).

edema (Figure 2A) as well as a highly vascularized 6.5  6.5  7.5 cm mass located in the left adrenal gland (Figure 2B). The patient was transferred to the intensive care unit where noninvasive bi-level positive airway pressure ventilation was initiated. Continuous renal replacement therapy and inotropic and vasopressor support using dobutamine and norepinephrine infusions were started. A transthoracic echocardiogram (TTE) displayed normal left ventricular (LV) dimensions, markedly reduced LV systolic function (visually estimated LV ejection fraction [LVEF], 15%-20%) with akinesia of the basal and mid LV segments although preserved apical wall motion, and mild mitral regurgitation (grade ¼; Video S1 [TTE], supplemental digital content). No significant pericardial effusion was observed. The diagnosis of inverted takotsubo-like cardiomyopathy from pheochromocytoma was presumed. Over the next hour, the patient’s condition further deteriorated, despite efforts to maintain adequate oxygenation and tissue perfusion. The hospital’s ECMO team was alerted prior to intubation, since cardiocirculatory collapse was anticipated and the underlying diagnosis considered treatable. Gum elastic bougie-assisted endotracheal intubation using ketamine, rocuronium, and alfentanil was uneventfully performed. Mechanical ventilation was started. Less than 20 minutes later, the patient abruptly developed severe bradycardia with deep ST-segment depression and profound hypotension unresponsive to atropine and epinephrine. Complete asystolic cardiac arrest ensued and cardiopulmonary resuscitation (CPR) was performed, while bedside percutaneous right femoral arterial (17F, 18 cm Bio-Medicus cannula [Medtronic Inc, Minneapolis, Minnesota]) and venous (23F, 50 cm Bio-Medicus cannula [Medtronic Inc]) cannulation using Seldinger technique for venoarterial ECMO took place. The patient was connected to the crystalloid-primed ECMO circuit (Thoratec CentriMag centrifugal pump [Thoratec Corp, Pleasanton, California], Quadrox PLS oxygenator [MAQUET

Cardiopulmonary AG, Hirrlingen, Germany]), and ECMO flow was started after approximately 30 minutes of continuous manual chest compression CPR. Subsequently, the patient was transferred to the ECMO unit. Sinus rhythm was restored and within 30 minutes of bypass initiation, the patient showed signs of life by opening eyes and squeezing hands on command. She was sedated with propofol, dexmedetomidine, and morphine. Heart rate was 50 to 100 beats/min with electromechanical dissociation (pulseless electrical activity); mean arterial blood pressure was 60 to 70 mm Hg on vasopressor support; pupils were dilated and unresponsive. Continuous venovenous hemodiafiltration was sustained. Targeted temperature management aimed at 34 C to 35 C for 48 hours was implemented for neuroprotection. A repeat TTE, 10 hours after the initial assessment, showed severe biventricular failure (LVEF, 0%) with global akinesia except for slight LV apical wall motion; transaortic flow had fully ceased (Video S2 [TTE], supplemental digital content). Levosimendan and milrinone infusions were introduced to diminish the risk of LV and pulmonary thrombus formation until myocardial contractility gradually improved starting by the end of hospital day 1. Laboratory findings suggested progressive acute liver dysfunction (peak international normalized ratio, 1.6; peak ALT, 10.46 mkat/L [reference range,

Pheochromocytoma-Induced Inverted Takotsubo-Like Cardiomyopathy Leading to Cardiogenic Shock Successfully Treated With Extracorporeal Membrane Oxygenation.

Pheochromocytoma classically displays a variety of rather benign symptoms, such as headache, palpitations, and sweating, although severe cardiac manif...
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